Paraneoplastic neurological syndromes (PNS) are rare presentations of an underlying oncological disease and more unusual during an oncological disease. They most likely present in small-cell lung carcinomas and thymomas, but present in <1% of the gynecological neoplasms. Acknowledging the pathophysiology is essential for management, explaining its clinical presentation, and future research. We present a patient with an underlying gynecological cancer that during her disease developed a PNS with an unusual autoantibody (anti-CV2/CRMP5) mediating the disease. We report a case of a 62-year-old female diagnosed with ovarian cancer who in the course of her disease developed neurological symptoms associated with cerebellar degeneration. After ruling out differential diagnoses such as metastases, a PNS was suspected and studied, in which anti-CV2/CRMP5 antibodies were positive. With her clinical presentation, radiological features, autoantibody positivity on cerebrospinal fluid, and an underlying oncological disease, cerebellar degeneration was diagnosed. The pathophysiology of PNS is not fully understood; therefore, its diagnosis and management are complex. Diagnosis is based on clinical presentation and specific antibodies associated. Unfortunately, patients have a bad prognosis and diminished quality of life, and therefore a multidisciplinary approach is needed. It is important to mention that the presentation of PNS does not mandatorily appear before the diagnosis of cancer, and multiple cases have been reported in which patients with an underlying oncological disease develop these syndromes. As medical oncologists and neurologists, we must consider and study these syndromes as a possible etiology in cases with an underlying cancer who develop neurological symptoms in the course of their disease.

No abstract

BackgroundParaneoplastic neurological syndromes are rare presentations of an underlying oncological disease and even more unusual they can present during an oncological disease. These syndromes more likely present in small cell lung carcinomas and thymomas, but in less than 1% of the cases gynecological neoplasms have shown this paraneoplastic presentation, such as the case presented. Even though not completely understood yet, acknowledging the pathophysiology is essential for management, relate other types of neoplasms and explain its clinical presentation. We present a patient with an underlying gynecological cancer, that during her disease developed a paraneoplastic neurological syndrome with an unusual autoantibody (anti-CV2/CRMP5) mediating the disease.Case PresentationA 62-yo female diagnosed with ovarian cancer who in the course of her disease develops neurological symptoms associated with cerebellar degeneration. After ruling out differential diagnoses such as metastases, a neurological paraneoplastic syndrome was suspected and studied, in which anti-CV2/CRMP5 were positive. Putting together her clinical presentation, radiological features, auto-antibody positivity on CSF related with paraneoplastic neurological syndromes and an underlying oncological disease, cerebellar degeneration as a paraneoplastic syndrome was diagnosed.ConclusionThe pathophysiology of neurological paraneoplastic syndromes is not fully understood; therefore, its diagnosis and management are complex. Diagnosis is based on clinical presentation and specific antibodies associated. Unfortunately, patients have a bad prognosis and diminished quality of life, therefore its management needs a multidisciplinary approach. It is important to mention that the presentation of paraneoplastic neurological syndromes do not mandatory appear before the diagnosis of cancer, multiple cases have been reported in which patients with an underlying oncological disease develop these syndromes. As medical oncologists and neurologists we must consider and study these syndromes as a possible etiology in cases with an underlying cancer who develop neurological symptoms in the course of their disease after ruling out differential diagnoses such as brain metastases.

The adamantinomas are rare tumours. It is important to recognise this type of tumor from the beginning, since its prognosis is excellent in initial stages. It is important to have free margins as survival is very high.